Airway management of a child with frontometaphyseal dysplasia (Gorlin Cohen syndrome)

Abstract

Frontometaphyseal dysplasia (FMD), also called Gorlin-Cohen syndrome, is a rare hereditary X-linked dominant craniotubular bone disorder. The presentation describes the airway management of a 2-year-old child suffering from FMD with significant retrognathia, posted for major long bone corrective osteotomy. Induction with a combination of dexmedetomidine and ketamine preceded a successful endotracheal intubation under spontaneous ventilation.

Keywords

• Dexmedetomidine
• difficult airway
• frontometaphyseal dysplasia
• ketamine